Changes in the Characteristics and Initial Treatments of Pulmonary Hypertension Between 2008 and 2020 in Japan

Tamura, Yuichi; Kumamaru, Hiraku; Inami, Toru; Matsubara, Hiromi; Hirata, Ken‐ichi; Tsujino, Ichizo; Suda, Rika; Matsubara, Hiroaki; Nishimura, Shiori; Sigel, Byron; Takano, Masashi

doi:10.1016/j.jacasi.2022.02.011

Cited by 14 publications

(25 citation statements)

References 36 publications

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“…This suggestion is supported by the fact that the baseline patient characteristics in the real-world setting substantially differed (i.e., identification at an older age, more males, higher percentage of patients with IPAH, and so on) from those in the PAH registry. 5,25 This study also indicated that CV risk was not significantly associated with all-cause mortality and PAH-related emergency hospitalization at the 0.05 level of significance. A previous study using the COMPERA registry indicated that the patients with multimorbidity IPAH resulted in no significant difference in overall survival.…”

Section: Prognostic Factors Of Survival and Pah-related Hospitalizationsmentioning

confidence: 64%

“…Nonetheless, our findings, compared to studies conducted at PAH centers, indicate that Japanese patients with PAH might have a more unfavorable prognosis. This suggestion is supported by the fact that the baseline patient characteristics in the real‐world setting substantially differed (i.e., identification at an older age, more males, higher percentage of patients with IPAH, and so on) from those in the PAH registry 5,25 …”

Section: Discussionmentioning

confidence: 98%

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Real‐world clinical practice of pulmonary arterial hypertension in Japan: Insights from a large administrative database

Omura,

Kitahara,

Takano

et al. 2023

Pulm. circ.

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Pulmonary arterial hypertension (PAH) is a fatal disease that often occurs at an early age. In recent years, aggressive treatment with multiple drugs from the early‐stage diagnosis is expected to improve the prognosis. Indeed, a high rate of initial combination therapy and excellent treatment outcomes have been reported from specialized centers for PAH in Japan. However, information on PAH epidemiology, including non‐PAH specialized centers in Japan, is unclear. To address the above, we conducted a retrospective observational cohort study from April 2008 to September 2020 using real‐world evidence from a large‐scale administrative database (Medical Data Vision) to examine baseline characteristics, comorbidities, and treatment profiles of Japanese patients with PAH. Five hundred and eighteen patients with PAH (treatment‐naive PAH, age 67.2 ± 15.9) were identified through our comprehensive approach which combined PAH disease codes, medications, and diagnostic procedures. Moreover, we showed that a larger proportion of patients received monotherapy in their initial treatment (66%) compared to those receiving combination therapy (34%). During the 1‐year follow‐up after PAH diagnosis, 13% of patients increased their PAH medications while other patients either decreased their PAH medications (6%) or discontinued PAH treatment (27%). The 3‐ and 5‐year event‐free survival rates of all‐cause death were 72% and 64%, respectively. This is the first large‐scale administrative database study that provides insights into real‐world PAH management in Japan. This study highlighted a different PAH clinical landscape which included a larger portion of the elderly population, higher initial monotherapy treatment, and lower survival rates than previous studies.

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Section: Prognostic Factors Of Survival and Pah-related Hospitalizationsmentioning

confidence: 64%

Section: Discussionmentioning

confidence: 98%

Real‐world clinical practice of pulmonary arterial hypertension in Japan: Insights from a large administrative database

Omura,

Kitahara,

Takano

et al. 2023

Pulm. circ.

Self Cite

View full text Add to dashboard Cite

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“…27 In a recent Japanese registry study, more than 60% of PoPH patients (22 patients) were prescribed combination therapy, with ERA+NO being the most common (36.4%). 28 Incidence of complications. The incidence of complications, except for malignancy, tended to be higher in PoPH group than portal hypertension w/o PAH group.…”

Section: Discussionmentioning

confidence: 99%

“…In Japan, there was a case report claiming that the combination of Ambisentan+Tadalafil was effective for PoPH, but more information was needed due to the small sample size 27 . In a recent Japanese registry study, more than 60% of PoPH patients (22 patients) were prescribed combination therapy, with ERA+NO being the most common (36.4%) 28 …”

Section: Discussionmentioning

confidence: 99%

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Treatment pattern and clinical outcomes in portopulmonary hypertension: A database study in Japan

Atsukawa

Takano²,

Omura³

2022

JGH Open

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Background and Aim Portopulmonary hypertension (PoPH) is a complication associated with portal hypertension. Since the epidemiological study in Japan was limited, this study aimed to investigate the characteristics, treatment patterns, and prognosis of PoPH patients in real‐world data. Methods The characteristics and treatment patterns of PoPH (n = 386) and portal hypertension without pulmonary arterial hypertension (portal hypertension w/o PAH) (n = 96 463) were analyzed based on the Medical Data Vision (MDV) dataset from April 2008 to September 2020. Survival‐time analyses of emergency hospitalization and mortality were also conducted between matched pair cohorts of PoPH (n = 210) and portal hypertension w/o PAH (n = 840). Results Among 386 PoPH patients, the Child–Pugh classification of PoPH group comprised patients with Class A (59 [15.3%]), B (109 [28.2%]), and C (42 [10.9%]), and missing (176 [45.6%]). Regarding the feature of PoPH group, the proportion of primary biliary cholangitis (PBC) (13.7%) and splenomegaly (9.8%) was higher compared with portal hypertension w/o PAH group. The survival time of all‐cause hospitalization in PoPH group was shorter than portal hypertension w/o PAH group in matched pair cohort (P < 0.001 by log‐rank test). Of PoPH patients, the frequency of PAH‐specific medicine usage within 90 days was monotherapy of 79 patients (20.5%), combination therapy of 64 patients (16.6%), and PAH‐specific medicine usage of 243 patients (63.0%). Conclusion This was the first study demonstrating that high proportion of PBC and splenomegaly and a greater risk of hospitalization were observed in PoPH patients based on the analysis using administrative claim database.

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SATISFY‐JP, a phase II multicenter open‐label study on Satralizumab, an anti‐IL‐6 receptor antibody, use for the treatment of pulmonary arterial hypertension in patients with an immune‐responsive‐phenotype: Study protocol

Tamura

Takeyasu

Takata³

et al. 2023

Pulm. circ.

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Pulmonary arterial hypertension (PAH), an intractable disease with a poor prognosis, is commonly treated using pulmonary vasodilators modulating the endothelin, cGMP, and prostacyclin pathway. Since the 2010s, drugs for treating pulmonary hypertension based on mechanisms other than pulmonary vasodilation have been actively developed. However, precision medicine is based on tailoring disease treatment to particular phenotypes by molecular‐targeted drugs. Since interleukin‐6 (IL‐6) is involved in the development of PAH in animal models, and some patients with PAH have elevated IL‐6 levels, the cytokine is expected to obtain potentials for therapeutic targeting. Accordingly, we identified a phenotype with elevated cytokine activity of the IL‐6 family in the PAH population by combining case data extracted from the Japan Pulmonary Hypertension Registry with a comprehensive analysis of 48 cytokines using artificial intelligence clustering techniques. Including an IL‐6 threshold ≥2.73 pg/mL as inclusion criteria for reducing the risk of insufficient efficacy, an investigator‐initiated clinical study using satralizumab, a recycling anti‐IL6 receptor monoclonal antibody, for patients with an immune‐responsive phenotype is underway. This study is intended to test whether use of patient biomarker profile can identify a phenotype responsive to anti‐IL6 therapy.

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Changes in the Characteristics and Initial Treatments of Pulmonary Hypertension Between 2008 and 2020 in Japan

Cited by 14 publications

References 36 publications

Real‐world clinical practice of pulmonary arterial hypertension in Japan: Insights from a large administrative database

Real‐world clinical practice of pulmonary arterial hypertension in Japan: Insights from a large administrative database

Treatment pattern and clinical outcomes in portopulmonary hypertension: A database study in Japan

SATISFY‐JP, a phase II multicenter open‐label study on Satralizumab, an anti‐IL‐6 receptor antibody, use for the treatment of pulmonary arterial hypertension in patients with an immune‐responsive‐phenotype: Study protocol

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